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Background: Recently, triglyceride deposit cardiomyovasculopathy (TGCV) with defective intracellular lipolysis was found to be a disease that causes heart failure. As a diagnostic criterion for TGCV, an Iodaine-123-β-methyl iodophenyl-pentadecanoic acid washout rate (BMIPP WOR) of < 10% is used, but its clinical significance in patients with heart failure remains to be clarified., Methods: In 62 hospitalized patients with chronic heart failure, 123 I-BMIPP myocardial single-photon emission computed tomography (SPECT) was performed predischarge state. The prevalence of TGCV was investigated. Subsequently, follow-up was conducted for ≥ 90 days (mean: 724.6 ± 392.7 days), and the association between the BMIPP WOR and cardiac events was examined, establishing all-cause mortality and admission due to heart failure as endpoints., Results: Of the 62 patients, the WOR was < 10% in 41 (66.1%). Of these, 26 (41.9%) were diagnosed with definite TGCV. Furthermore, cardiac events were noted in 12 patients (19.4%). Analysis with Cox proportional hazards models showed that the BMIPP WOR < 4.5% was a significant event-predicting factor [HR 4.29, 95% CI: 1.20-16.87; p = 0.0245]. On a Kaplan-Meier curve, the WOR was 4.5%; there was a significant difference in the incidence of events (p = 0.0298)., Conclusion: In the predischarge state of heart failure, 123 I-BMIPP myocardial SPECT was performed. In approximately 40% of the patients, a diagnosis of TGCV was made. The results suggested that the BMIPP WOR is useful for predicting the prognosis of chronic heart failure patients regardless of TGCV., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Little is known about the prognostic impact of heart failure (HF) duration in patients with advanced HF., Methods: A total of 109 consecutive patients with advanced HF referred to the institutional heart transplant program between July 2014 and December 2017 were prospectively enrolled. The patients were divided into two groups according to the HF duration using a pre-specified cutoff (> 18 months, n = 38; ≤ 18 months, n = 71). The Cox proportional hazards model was generated to investigate the association between the HF duration and a 1-year composite endpoint (all-cause mortality, left ventricular assist device implantation, and hospitalization due to HF)., Results: Patients with a longer HF duration were older and had significantly lower blood pressure, and greater left ventricular volume compared with those with a shorter HF duration. The 1-year event-free survival rate was significantly lower in patients with a longer HF duration (49.1% vs. 80.0%, log-rank p < 0.001). After adjustment, a longer HF duration was independently associated with an increased risk for the composite endpoint (hazard ratio, 2.44; 95% confidence interval, 1.03-5.76; p = 0.04). Additionally, longer HF duration was independently associated with an increased wall motion score index and a decreased heart-to-mediastinum ratio of 123 I-meta-iodobenzylguanidine (MIBG) myocardial scintigraphy (all associations, p < 0.05)., Conclusions: A longer HF duration is associated with an increased risk of adverse outcomes as well as more severe myocardial damage among patients with advanced HF.

The article discusses the distinct pathways of myocardial triglyceride (TG) lipolysis in primary and idiopathic triglyceride deposit cardiomyovasculopathy (TGCV), a rare cardiovascular disorder associated with heart failure. In TGCV, defective intracellular TG lipolysis leads to TG deposition and energy failure in cardiomyocytes and coronary smooth muscle cells. The study analyzes endomyocardial biopsy specimens from TGCV patients and finds that in primary TGCV, the defect lies in ATGL-dependent lipolysis, while in idiopathic TGCV, an ATGL-independent lipolysis pathway may be involved. These findings contribute to understanding the heterogeneity of myocardial TG lipolysis pathways and the complex regulatory mechanisms for lipid droplet formation in heart failure. [Extracted from the article]

Background: Recently, triglyceride deposit cardiomyovasculopathy (TGCV) with defective intracellular lipolysis was found to be a disease that causes heart failure. As a diagnostic criterion for TGCV, an Iodaine-123-β-methyl iodophenyl-pentadecanoic acid washout rate (BMIPP WOR) of < 10% is used, but its clinical significance in patients with heart failure remains to be clarified. Methods: In 62 hospitalized patients with chronic heart failure, 123I-BMIPP myocardial single-photon emission computed tomography (SPECT) was performed predischarge state. The prevalence of TGCV was investigated. Subsequently, follow-up was conducted for ≥ 90 days (mean: 724.6 ± 392.7 days), and the association between the BMIPP WOR and cardiac events was examined, establishing all-cause mortality and admission due to heart failure as endpoints. Results: Of the 62 patients, the WOR was < 10% in 41 (66.1%). Of these, 26 (41.9%) were diagnosed with definite TGCV. Furthermore, cardiac events were noted in 12 patients (19.4%). Analysis with Cox proportional hazards models showed that the BMIPP WOR < 4.5% was a significant event-predicting factor [HR 4.29, 95% CI: 1.20–16.87; p = 0.0245]. On a Kaplan–Meier curve, the WOR was 4.5%; there was a significant difference in the incidence of events (p = 0.0298). Conclusion: In the predischarge state of heart failure, 123I-BMIPP myocardial SPECT was performed. In approximately 40% of the patients, a diagnosis of TGCV was made. The results suggested that the BMIPP WOR is useful for predicting the prognosis of chronic heart failure patients regardless of TGCV. [ABSTRACT FROM AUTHOR]

Triglyceride deposit cardiomyovasculopathy (TGCV) is a phenotype primarily reported in patients carrying genetic mutations in PNPLA2 encoding adipose triglyceride lipase (ATGL) which releases long chain fatty acid (LCFA) as a major energy source by the intracellular TG hydrolysis. These patients suffered from intractable heart failure requiring cardiac transplantation. Moreover, we identified TGCV patients without PNPLA2 mutations based on pathological and clinical studies. We provided the diagnostic criteria, in which TGCV with and without PNPLA2 mutations were designated as primary TGCV (P-TGCV) and idiopathic TGCV (I-TGCV), respectively. We hereby report clinical profiles of TGCV patients. Between 2014 and 2018, 7 P-TGCV and 18 I-TGCV Japanese patients have been registered in the International Registry. Patients with I-TGCV, of which etiologies and causes are not known yet, suffered from adult-onset severe heart disease, including heart failure and coronary artery disease, associated with a marked reduction in ATGL activity and myocardial washout rate of LCFA tracer, as similar to those with P-TGCV. The present first registry-based study showed that TGCV is an intractable, at least at the moment, and heterogeneous cardiovascular disorder. [ABSTRACT FROM AUTHOR]

Introduction: Triglyceride deposit cardiomyovasculopathy (TGCV) is a novel clinical entity that was discovered in Japanese patients waiting for heart transplant. TGCV is characterized by the accumulation of triglycerides in the myocardium and coronary artery caused by intracellularly impaired fatty acid metabolism. Hence, the myocardial washout rate (WOR) of iodine-123-beta-methyl iodophenyl-pentadecanoic acid (BMIPP) plays an important role for the diagnostic criteria for TGCV. However, executing the early and delayed imaging is burdensome because of its procedure time. Therefore, we examined to diagnose patients with TGCV using only the early imaging of BMIPP scintigraphy. Hypothesis: The early imaging of BMIPP can detect the impaired fatty acid metabolism in patients with TGCV. Methods: We performed BMIPP and 201Thallium (Tl) scintigraphy in 169 patients with cardiac disease to measure the total counts of Tl and BMIPP in the early and delayed phase. All patients were applied to diagnostic criteria for TGCV. To assess the fatty acid metabolism, we calculated early BMIPP indices, the heart to mediastinum ratio (H/M) of BMIPP and the relative accumulation of BMIPP to Tl (BMIPP/Tl) in the early phase, and the diagnostic values of the early indices and the WORs of BMIPP were evaluated by ROC analysis. Results: Among 169 patients, 74 received the definitive diagnosis of TGCV. The diagnostic values of the BMIPP indices were evaluated by ROC analysis. Area under the curve (AUC) of H/M, BMIPP/Tl and WOR of BMIPP were 0.516 (95% confidence interval(CI): 0.418- 0.613), 0.744 (95%CI: 0.667-0.821) and 0.873 (95%CI: 0.820-0.927) respectively. In early indices, the AUC of BMIPP/Tl is significantly larger than that of H/M (p < 0.05). Conclusions: For the diagnosis of TGCV, reduced WOR of BMIPP is a crucial index. Although H/M cannot replace the WOR, BMIPP/Tl uptake ratio would be useful to some extent to detect the impaired fatty acid metabolism in patients with TGCV. [ABSTRACT FROM AUTHOR]